Mitochondria “Shackled” by Mutant Huntingtin: Analysis of Morphological Alterations and Disruptions of Intracellular Transport

Pasko, V.I.; Churkina, A.S.; Belikova, L.D.; Shakhov, A.S.; Lavrushkina, S.V.; Burakov, A.V.; Bogomazova, A.N.; Lagarkova, M.A.; Alieva, I.B.

Авторы: Pasko Vyacheslav I., Churkina Aleksandra S., Belikova Lilia D., Shakhov Anton S., Lavrushkina Svetlana V., Burakov Anton V., Bogomazova Alexandra N., Lagarkova Maria A., Alieva Irina B.
Журнал: Biochemistry (Moscow)
Том: 91
Номер: 2
Год издания: 2026
Издательство: Pleiades Publishing, Ltd
Местоположение издательства: Road Town, United Kingdom
Первая страница: 253
Последняя страница: 273
DOI: 10.1134/s0006297925602850
Аннотация: Mitochondria are semi-autonomous, multifunctional organelles that supply cells with energy. Theyare highly dynamic structures, capable of moving, fusing, dividing, and forming branched networks. Thenumber, density, and complexity of mitochondrial network are unique to each cell type and reflect cellulardemands for ATP and other mitochondria-dependent metabolites. Mitochondrial dysfunction is a hallmark ofmany neurodegenerative diseases; however, the relationships between neurodegeneration and mitochondrialmorphogenesis, intracellular localization, and dynamics remain incompletely understood. Interpretation andcomparison of published data are complicated by the diversity of analytical approaches used to study mito-chondrial behavior. In this research, we investigated the effects of a pathogenic mutation in the huntingtinprotein (HTT), which causes Huntington’s disease (HD), on mitochondrial morphology and motility, withparticular emphasis on associated disruptions in the cytoskeletal organization. We performed a systematicevaluation of automated mitochondrial analysis tools and selected MiNA, TrackMate, and JACoP as theoptimal platforms for quantitative assessment of the effects of mutant HTT (mHTT) on the mitochondrialmorphology, motility, and interaction with cytoskeletal components and identification of specific disruptionsdirectly related to HD pathogenesis. Our analysis revealed that mitochondria in mHTT-expressing cells aresignificantly shorter, more branched, and less motile than in control cells. Moreover, their interactions withmicrotubules and vimentin intermediate filaments are markedly altered. Together, these findings establisha link between HD and specific defects in the mitochondrial network, thus contributing to understandingcellular mechanisms of HD development, and suggest that mHTT disrupts the interaction of mitochondriawith cytoskeletal components responsible for their movement and distribution in the cell, thereby negativelyaffecting mitochondrial motility and morphology.
Добавил в систему: Чуркина Александра Сергеевна

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Mitochondria “Shackled” by Mutant Huntingtin: Analysis of Morphological Alterations and Disruptions of Intracellular Transportстатья

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